When a Bleeding Disorder Causes a Deep Hemorrhage

Managing a large chest-wall hematoma in type 2B von Willebrand disease

By William C. Aird, MD

Case Presentation

A 50-year-old woman with a historical diagnosis of type 2B von Willebrand disease (VWD) presents with four days of progressively worsening right posterior chest and back pain.

She recalls no major trauma, although she had recently reached overhead, walked a large dog that occasionally pulled on its leash, and underwent a deep tissue massage after the pain began.

Because of severe pain, she presents to an outside emergency department.

CT angiography demonstrates a 16-cm hematoma involving the right chest wall near the serratus anterior muscle with active contrast extravasation.

Initial laboratory studies show:

She receives tranexamic acid and desmopressin before transfer to a tertiary care center.

Despite the imaging findings, she remains hemodynamically stable.

Bleeding History

She has experienced lifelong easy bruising and heavy menstrual bleeding.

During delivery of twins she required desmopressin after postpartum hemorrhage.

All three of her daughters have also been diagnosed with type 2B VWD.

She has never previously experienced a major spontaneous soft-tissue hemorrhage.

Which feature of this presentation is most concerning?

Explanation

Large intramuscular or chest-wall hematomas are uncommon in VWD but may occur.

The combination of:

• a large expanding hematoma
• active contrast extravasation
• declining hemoglobin

suggests clinically important bleeding requiring close monitoring and targeted hemostatic therapy.

Although PT and aPTT are normal, normal coagulation screening tests do not exclude clinically significant VWD.

Diagnostic Background

Historical evaluation had established a diagnosis of type 2B VWD.

Laboratory studies obtained during this admission demonstrated:

The ratio of platelet-dependent VWF activity to VWF antigen is markedly reduced, consistent with qualitative VWF dysfunction.

These studies support a type 2 VWD phenotype but do not independently establish the subtype.

Which laboratory finding is most consistent with a qualitative defect in von Willebrand factor?

Explanation

Patients with type 2 VWD often have:

• relatively preserved VWF antigen
• disproportionately reduced platelet-dependent VWF activity

The activity-to-antigen ratio therefore falls below normal, reflecting impaired VWF function rather than decreased quantity.

Initial Management

Interventional radiology reviews the CT scan.

Although active contrast extravasation is present, the patient remains:

• normotensive
• without tachycardia
• clinically stable

Compression is applied over the hematoma, and serial hemoglobin measurements are obtained.

Hemoglobin falls further:

Because of continued bleeding, she receives 6,000 units of Humate-P, a plasma-derived concentrate containing both VWF and factor VIII.

Why is desmopressin generally avoided in patients with type 2B VWD who develop major bleeding?

Explanation

Desmopressin releases endogenous VWF from endothelial storage sites.

In type 2B VWD, the released VWF has increased affinity for platelet glycoprotein Ib.

This may:

• enhance platelet binding
• precipitate thrombocytopenia
• provide less effective hemostasis than VWF replacement

For major bleeding, current practice generally favors VWF-containing concentrate rather than desmopressin.

Clinical Course

Following administration of Humate-P, the patient’s hemoglobin stabilizes.

Serial examinations demonstrate no further expansion of the hematoma.

Because she remains clinically stable, interventional radiology determines that angiographic embolization is unnecessary.

She is discharged home with hematology follow-up and repeat CBC monitoring.

Which factor most strongly supported continued conservative management rather than immediate embolization?

Explanation

Active contrast extravasation does not automatically require embolization.

Management should integrate:

• hemodynamic status
• hemoglobin trajectory
• clinical examination
• response to hemostatic therapy
• feasibility of local intervention

In this patient, stabilization after VWF replacement and compression allowed successful nonoperative management.

Teaching Points

• Type 2B VWD is a qualitative disorder of VWF function rather than VWF quantity.
• Normal PT, aPTT, and even a normal platelet count do not exclude clinically significant type 2B VWD
• A reduced platelet-dependent VWF activity relative to VWF antigen suggests qualitative VWF dysfunction.
• For major bleeding in type 2B VWD, VWF-containing concentrate is generally preferred over desmopressin.
• Management of major bleeding requires integration of clinical status, laboratory trends, imaging findings, and disease-specific hemostatic therapy.

Why This Case Matters

Von Willebrand disease is often thought of as a disorder of mucosal bleeding, but major soft-tissue hemorrhage can occur and requires a different level of clinical urgency.

This case illustrates three important principles. First, laboratory interpretation depends on assessing VWF function as well as quantity. Second, identifying the specific VWD subtype influences treatment decisions, particularly the choice between desmopressin and VWF replacement. Finally, successful management of major bleeding requires more than interpreting imaging findings. Decisions about intervention depend on the patient’s overall clinical trajectory, emphasizing that treatment is guided by the patient, not the CT scan.